AffiAB® Anti-NLRP3 Antibody

NLR family pyrin domain containing 3 (NLRP3) (previously known as NACHT, LRR and PYD domains-containing protein 3 [NALP3] and cryopyrin) , is a protein that in humans is encoded by the NLRP3 gene located on the long arm of chromosome 1. NLRP3 is expressed predominantly in macrophages and as a component of the inflammasome, products of damaged cells such as extracellular ATP and crystalline uric acid. Activated NLRP3 in turn triggers an immune response. Mutations in the NLRP3 gene are associated with a number of organ specific autoimmune diseases. Cryopyrin interacts selectively with apoptosis associated specklike protein containing a CARD domain (ASC) . This complex may function as an upstream activator of NFkB signaling and caspase-1 activation. The complex also inhibits TNFa induced activation and nuclear translocation of RelA/NFkB p65. Mutations in Cryopyrin and Pyrin proteins are responsible for several autoinflammatory disorders in humans, including familial cold autoinflammatory syndrome (FCAS) , Muckle-Wells syndrome (MWS) and chronic infantile neurologic cutaneous and articular syndrome (CINCA) .